Reducing Fatty Acid Oxidation Improves Cancer-free Survival in a Mouse Model of Li-Fraumeni Syndrome.

ABSTRACT: Germline mutations of TP53, which cause the cancer predisposition disorder Li-Fraumeni syndrome (LFS), can increase mitochondrial activity as well as fatty acid β-oxidation (FAO) in mice. Increased fatty acid metabolism can promote cancer malignancy, but its specific contribution to tumorigenesis in LFS remains unclear. To investigate this, we crossed LFS mice carrying the p53 R172H knock-in mutation (p53^172H/H , homolog of the human TP53 R175H LFS mutation) with myoglobin-knockout (MB^-/- ) mice known to have decreased FAO. MB^-/- p53^172H/H double-mutant mice also showed mildly reduced FAO in thymus, a common site of T lymphoma development in LFS mice, in association with an approximately 40% improvement i

SUBMITTER: Wang PY 

PROVIDER: S-EPMC7981282 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature